Journal of JCIC

We report the case of a female infant with complex congenital heart disease who encountered significant challenges during modified Blalock–Taussig shunt (MBTS) embolization. The patient was a 4-month-old female, diagnosed with asplenia, dextrocardia, right ventricular single ventricle, single atrium, a common atrioventricular valve, valvular and subvalvular pulmonary stenosis, total anomalous pulmonary venous return (cardiac type), a right aortic arch, and bilateral superior vena cava. At 39 days of life (body weight: 3.49 kg), she underwent MBTS surgery using a 3.5-mm expanded polytetrafluoroethylene graft due to recurrent hypoxic episodes. However, pulmonary overcirculation complicated postoperative care, resulting in difficulty in controlling heart failure. Poor general condition due to shock state, made open-heart surgery unfeasible. Percutaneous embolization was planned to provide emergency and minimally invasive management, and the procedure was performed on day 101. The acute angle between the subclavian artery and the MBTS anastomosis posed a technical challenge, rendering catheter and long sheath insertion difficult. Despite these difficulties, embolization was successfully achieved using a 4-mm Amplatzer™ Vascular Plug I for the MBTS (internal diameter: 2.6 mm), placing a left coronary artery catheter in the peripheral pulmonary artery along with a stiff wire, and advancing the long sheath. It was useful to select a catheter that matched the shape of the pulmonary artery and advance a long sheath while it was in place along with a stiff wire. However, in cases with smaller body sizes, careful manipulation is necessary due to the risk of vascular injury.